부산대학교 도서관

A 7 year old Nigerian girl was referred by Haematology to the Paediatric Dermatology team with "suspected eczema". She had known sickle cell disease, for which she was on hydroxycarbamide, Mum reported an 8 month history of a new rash with no systemic features. She had marked indurated plaques on face, forehead and forearms which were associated with scarring and hyperpigmentation. She had a number of investigation including negative urine dipstick. ANA was positive with elevated RO and RNP antibodies. Inflammatory markers were normal. Skin biopsy results were consistent with discoid lupus erythematosus. Stringent photo protection and sun blocking creams were advised. She was commenced on potent topical steroids and hydroxychloroquine which is first line systemic treatment. Within 4 weeks of commencing hydroxychloroquine there was significant flattening of the plaques. She was referred to Paediatric Rheumatology for assessment, given her high risk features. Clinical features of DLE in children are similar to adult DLE in presentation and chronic course. However it is rare in children with less than 2% of patients developing DLE before 10 years of age. DLE tends to be more common and severe in skin of colour. There are complications of scarring and pigmentation. It is important to be aware of the psychological impact on both parent and child's quality of life. There is high risk of transition to SLE in children with DLE and hence screening bloods and early referral to Rheumatology should be considered. [ABSTRACT FROM AUTHOR]